OBJECTIVETo explorer the clinical features, diagnosis and therapy of Langerhans cell sarcoma (LCS).

METHODSThe clinical data of a case of LCS originated from cervical lymph nodes was analyzed. The pathological biopsy was studied by cell morphology, immunohistochemistry and electron microscopy, and the related literature was reviewed.

RESULTSThe giant tumor cells were characterized by markedly malignant proliferation, irregular nuclei and obviously chromatin abnormality, the positive S-100, CD1a and Langerin (CD207) tumor cells were revealed by immunohistochemistry, and Birbeck granules could be found by electron microscopy. All of them supported the diagnosis of LCS. The patient's condition progressed rapidly and died of multiple organ failure in a short time.

CONCLUSIONLCS is an extremely rare neoplastic proliferation of Langerhans cells with overtly malignant cytologic features and spreads aggressively. The diagnosis of LCS mainly relies on pathological cell morphology, immunohistochemistry and electron microscopy if necessary. The treatment includes chemotherapy, surgery and radiotherapy, etc, but lack of generally accepted optimal treatment regimen currently. In short, LCS has intensive invasiveness and poor prognosis.

Langerhans cell sarcoma is a rare, proliferative tumor of Langerhans cells, which shows cytologic characteristics and clinical features of malignant tumor. Langerhans cell sarcoma primarily occurs in lymph nodes, skin, lung, liver, and spleen. However, very few cases have been reported in the head and neck region. Because of its rarity, an optimal treatment approach is unknown; however, Langerhans cell sarcoma grows aggressively and shows a poor prognosis, such that a more aggressive and multi-modality treatment approach is necessary. Here, we report the case of a 36-year-old male with Langerhans cell sarcoma, who presented with a mass in the submandibular gland area and was treated with wide excision and postoperative radiotherapy.
Adult ; Head ; Humans ; Langerhans Cell Sarcoma ; Langerhans Cells ; Liver ; Lung ; Lymph Nodes ; Male ; Neck ; Prognosis ; Radiotherapy ; Sarcoma ; Skin ; Spleen ; Submandibular Gland

We describe a case of cavitary metastasis to the lungs from a small angiosarcoma of the scalp, in which themetastatic lesions were complicated by pneumothorax and pulmonary hemorrhage. On high-resolution CT, the lesionssimulated the findings of Langerhans cell histiocytosis. Thin-walled cavitary metastatic lesions were similar tothose of thin walled air cysts in Langerhans cell histiocytosis. Ground-glass opacity simulated the findings ofsmoke r's respiratory bronchiolitis in Langerhans cell histio-cytosis but histologically represented hemorrhageduring metastasis of the angiosarcoma.
Bronchiolitis ; Hemangiosarcoma* ; Hemorrhage ; Histiocytosis, Langerhans-Cell ; Lung* ; Neoplasm Metastasis ; Pneumothorax ; Sarcoma ; Scalp

We report a case of Langerhans cell sarcoma presented as a solitary mass in the left supraclavicular area in a 31-year-old woman. Computed tomography revealed a relatively well-defined and lightly enhancing mass in the left supraclavicular area, measuring 5.5x4.5x3.2 cm. Excision was subsequently performed. Microscopically, the specimen consisted of an enlarged and partially effaced lymph node. Nests of different size composed of atypical tumor cells were located in the paracortex and the medulla of the lymph node. The tumor cells exhibited abundant eosinophilic or clear cytoplasm and displayed marked nuclear atypia and increased mitotic figures. Infiltration of many eosinophils was identified in the periphery and between the tumor cells. The tumor cells were reactive for CD1a and S100 protein. Ultrastructually, they were found to have Birbeck granules in the cytoplasm.
Adult ; Antigens, CD1 ; Cytoplasm ; Eosinophils ; Female ; Humans ; Langerhans Cell Sarcoma ; Lymph Nodes

Dendritic Cells ; pathology ; Histiocytes ; pathology ; Histiocytosis, Langerhans-Cell ; pathology ; Humans ; Keratins ; analysis ; Langerhans Cells ; pathology ; Lymphoma, Large B-Cell, Diffuse ; pathology ; Sarcoma ; chemistry ; classification ; pathology

Currently, the World Health Organization classifies Langerhans cell tumors into Langerhans cell histiocytosis and Langerhans cell sarcoma (LCS). LCS is a neoplastic proliferation of Langerhans cells showing malignant cytological features and aggressive clinical behavior with grave prognosis. Only a few cases have been reported in the available literature; therefore, to date, no definitive treatment has been established. A 64-year-old woman presented with a 1-year history of an asymptomatic, slow-growing erythematous nodule measuring 0.7 cm on her scalp. The patient also reported a 3-month history of a painful swelling on the right side of her neck. Histopathological examination of a scalp biopsy specimen revealed sheets of atypical cells with hyperchromatic nucleoli and clear cytoplasm. Immunohistochemical studies revealed malignant cells positive for CD1a, CD31, CD68, and S-100 expression. Additionally, positron emission tomography–computed tomography and fine-needle aspiration revealed LCS of the cervical lymph nodes and surrounding soft tissue. We recommended surgical excision and adjunctive chemotherapy; however, the patient refused treatment and died of the disease 28 months later.
Biopsy ; Biopsy, Fine-Needle ; Cytoplasm ; Drug Therapy ; Electrons ; Female ; Histiocytosis, Langerhans-Cell ; Humans ; Langerhans Cell Sarcoma ; Langerhans Cells ; Lymph Nodes ; Middle Aged ; Neck ; Parotid Gland ; Prognosis ; Scalp ; World Health Organization

The effect of radiation therapy, either alone or combined with surgery or chemotherapy is accepted well in the treatment of metastatic carcinoma, multiple myeloma, reticulum cell sarcoma and Ewing's sarcoma. But its effect on osteosarcoma and chondrosarcoma is less clear. The authors reviewed 90 patients treated with radiation therapy, 62 cases with primary bone tumor and 28 cases with multiple myeloma, from 1969 to 1988. There were 20 Ewing's sarcoma, 12 osteosarcoma, 12 chondrosarcoma, 9 Histiocytosis-X and 3 reticulum cell sarcoma among 62 primary bone tumors. And 40 patients with more than three months follow-up were analyzed for the primary response of tumor three months after radiation therapy and the long term effect of the radiation therapy. When the radiation therapy was done alone, the primary response was poor in osteogenic sarcoma, chondrosarcoma, Ewing's sarcoma and reticulum cell sarcoma. But with the multimodal therapy, the primary response was rslatively good in reticulum cell sarcoma and chondrosarcoma. In 15 patients, more than one year follow-up was done. The status of these patients at the last follow-up was poor in all cases trearted with radiation therapy alone than the multimodal therapy except Histiocytosis-X. In multiple myeloma, the effect of radiation therapy for the relief of pain was analyzed. There were complete relief of pain in 14.3%, partial relief in 71.4% and no relief in 10.7%.
Chondrosarcoma ; Drug Therapy ; Follow-Up Studies ; Histiocytosis, Langerhans-Cell ; Humans ; Lymphoma, Non-Hodgkin ; Multiple Myeloma ; Osteosarcoma ; Sarcoma, Ewing

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomographic (CT) scan revealed multiple tiny nodules in both lungs. The thoracoscopic lung biopsy revealed LCH. The patient quit smoking, but he received no other specific treatment. One year later, the follow up chest CT scan showed a 4 cm-sized mass in the left lower lobe of the lung. A lobectomy was then performed. Microscopic examination of the mass revealed an infiltrative proliferation of large cells that had malignant cytologic features. Immunohistochemical stains showed a strong reactivity for S-100 and CD68, and a focal reactivity for CD1a. We think this is the first case of LCS arising from LCH.
Tomography, X-Ray Computed ; Sarcoma/*pathology ; S100 Proteins/biosynthesis ; Radiography, Thoracic ; Pancreatic Neoplasms/*pathology ; Male ; Langerhans Cells/*pathology ; Immunohistochemistry ; Humans ; Histiocytosis, Langerhans-Cell/diagnosis/*pathology ; Gene Expression Regulation, Neoplastic ; Antigens, Differentiation, Myelomonocytic/biosynthesis ; Antigens, CD1/biosynthesis ; Antigens, CD/biosynthesis ; Adult

Langerhans cell histiocytosis (LCH) is a pleomorphic disease entity characterized by local or disseminated atypical Langerhans cells (LCs) found most commonly in bone, lungs, mucocutaneous structures and endocrine organs. Among different sites, unifocal disease confined to a lymph node (LN) is rare. We report a case of LCH confined to a LN in a 38-year-old male who presented with a 2 cm-sized cervical mass. The fine needle aspiration (FNA) smears of cervical LN showed high cellularity having isolated LCs with contorted nuclei and nuclear grooves mixed in multinucleated giant cells, small lymphocytes and eosinophils. Charcot-Leyden crystals were also seen, as were a few dendritic-like cells and intranuclear inclusions. Confirmation of LCH was made by histopathologic studies, positive reactions for S-100 protein and CD1a immunohistochemical staining and by the demonstration of Birbeck granules on electron microscopy. The differentials to be considered include dermatopathic lymphadenitis, sinus histiocytosis with massive lymphadenopathy, Hodgkin's lymphoma and malignant histiocytosis. The characteristic cytomorphologic pattern of LCH in a LN FNA smear plays an important role in suggesting the diagnosis of LCH.
Adult ; Biopsy, Fine-Needle* ; Diagnosis ; Eosinophils ; Giant Cells ; Histiocytic Sarcoma ; Histiocytosis, Langerhans-Cell* ; Histiocytosis, Sinus ; Hodgkin Disease ; Humans ; Intranuclear Inclusion Bodies ; Langerhans Cells ; Lung ; Lymph Nodes* ; Lymphadenitis ; Lymphocytes ; Male ; Microscopy, Electron ; S100 Proteins

PURPOSE: We evaluated the standard uptake value (SUV) of F-18 FDG at PET/CT for differentiation of benign from malignant tumor in primary musculoskeletal tumors. MATERIALS AND METHODS: Forty-six tumors (11 benign and 12 malignant soft tissue tumors, 9 benign and 14 malignant bone tumors) were examined with F-18 FDG PET/CT (Discovery ST, GE) prior to tissue diagnosis. The maxSUV(maximum value of SUV) were calculated and compared between benign and malignant lesions. The lesion analysis was based on the transverse whole body image. The maxSUV with cutoff of 4.1 was used in distinguishing benign from malignant soft tissue tumor and 3.05 was used in bone tumor by ROC curve. RESULTS: There was a statistically significant difference in maxSUV between benign (n=11; maxSUV 3.4+/-3.2) and malignant (n=12; maxSUV 14.8+/-12.2) lesions in soft tissue tumor (p=0.001). Between benign bone tumor (n=9; maxSUV 5.4+/-4.0) and malignant bone tumor (n=14; maxSUV 7.3+/-3.2), there was not a significant difference in maxSUV. The sensitivity and specificity for differentiating malignant from benign soft tissue tumor was 83% and 91%, respectively. There were four false positive malignant bone tumor cases to include fibrous dysplasia, Langerhans-cell histiocytosis (n=2) and osteoid osteoma. Also, one false positive case of malignant soft tissue tumor was nodular fasciitis. CONCLUSION: The maxSUV was useful for differentiation of benign from malignant lesion in primary soft tissue tumors. In bone tumor, the low maxSUV correlated well with benign lesions but high maxSUV did not always mean malignancy.
Body Image ; Bone Neoplasms ; Diagnosis ; Fasciitis ; Histiocytosis, Langerhans-Cell ; Osteoma, Osteoid ; Positron-Emission Tomography and Computed Tomography* ; ROC Curve* ; Sarcoma ; Sensitivity and Specificity ; Soft Tissue Neoplasms